International Delphi-based consensus: therapeutic approaches for pediatric adrenocortical carcinoma.
СтатьяConsensus StatementНазвание и аннотация: Перевод в очереди
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Maria Fragoso, de Ven Kees Van, Maria Riedmeier, Simone Abib, Shipra Agarwal, Perazollo Antoniazzi Augusto, Sonir Antonini, Suren Arul G, Fredrik Baecklund, Silvia Brandalise, Martin Daiggi Camila Maria, Cheri Deal, Jaydira Del Rivero, Tibor Denes Francisco, Katja Dumic, Saniye Ekinci, Tatiana El Jaick, Gernot Engstler, Martin Fassnacht, Cavalcante Figueiredo Bonald, Paula Flores, Daniela Fortunati, Laura Galuzzo, Nazlı Gönç, Wojciech Górecki, Gabriela Guercio, Melis Gültekin, Tulay Guran, Harm Haak, Emilie Hendriks, Jan Idkowiak, Dominika Janus, Vilani Kremer, Johanna Krueger, Lam Catherine G, Lopes Luiz Fernando, David Malkin, Despoina Manousaki, Celeste Mattone Maria, Stephen Marks, Carlos Molina, Jessica Munarin, Akihiro Ohmoto, Christina Pamporaki, Mara Pianovski, Nelson Piché, Nancy Perier, Raul Ribeiro, Chaves Ribeiro Rodrigo, Karla Rodrigues, Anna Taczanowska-Niemczuk, Thompson Lester D R, Gerdi Tuli, Max Van Noesel, Virgone Calogero, Waguespack Steven G, Martin Walz, Jonathan Wasserman, Armin Wiegering, Bilgehan Yalcin, Ronald R de Krijger, Verena Wiegering
Аннотация
Adrenocortical carcinoma (ACC) in children is a rare and aggressive malignancy. Despite representing a small fraction of pediatric adrenal tumors, ACC poses distinct challenges due to its heterogeneous biological behavior, frequent hormone secretion, and the limited availability of standardized, pediatric-specific treatment protocols. Complete surgical resection remains the cornerstone of curative intent therapy, and complete adrenalectomy offers the best chance for long-term survival. Given the aggressiveness of ACC, particularly in advanced disease, adjuvant therapies are often considered to improve outcomes. The aim of this consensus meeting was to bring together international experts to develop international guidance based on the best available evidence and collective clinical experience to harmonize long-term management. A modified 3-step Delphi consensus method was used to finalize the statements from an international panel of pediatric ACC experts from 4 continents (Europe, the Americas, and Asia, and Oceania). An electronic survey was developed by the steering committee (ENSAT Kids) and distributed to the panel. Agreement and disagreement were rated using a 6-point Likert scale with the opportunity to provide structured feedback. Consensus was defined a priori as ≥75% agreement. This Delphi process yielded consensus statements for therapy guidance, comprising 12 surgical statements and 68 medical treatment statements, organized by strategy and disease stage. These statements are intended to support clinicians in delivering more consistent and standardized therapeutic strategies for pediatric ACC. Future efforts should prioritize the design and conduct of high-quality, collaborative research studies in order to provide more evidence and therapy improvements.